Background: Cystinosis is a rare autosomal recessive lysosomal storage disease with high morbidity and mortality. It is caused by mutations in the CTNS gene that encodes the cystine transporter, cystinosin, which leads to lysosomal cystine accumulation. It is the major cause of inherited Fanconi syndrome, and should be suspected in young children with failure to thrive and signs of renal proximal tubular damage. The diagnosis can be missed in infants, because not all signs of renal Fanconi syndrome are present during the first months of life. Elevated white blood cell cystine content is the cornerstone of the diagnosis. Since chitotriosidase (CHIT1 or chitinase-1) is mainly produced by activated macrophages both in normal and inflammator

Massive multiple-input multiple-output (massive-MIMO) is a promising technology for next generation wireless communications systems due to its capability to increase the data rate and meet the enormous ongoing data traffic explosion. However, in non-reciprocal channels, such as those encountered in frequency division duplex (FDD) systems, channel state information (CSI) estimation using downlink (DL) training sequence is to date very challenging issue, especially when the channel exhibits a shorter coherence time. In particular, the availability of sufficiently accurate CSI at the base transceiver station (BTS) allows an efficient precoding design in the DL transmission to be achieved, and thus, reliable communication systems can be obtaine

Many waste materials can be repurposed effectively within asphalt concrete to enhance the performance and sustainability of pavement. One of these waste materials is sawdust ash (SDA). This study explores the beneficial use of SDA as a substitute for limestone dust (LD) mineral filler in asphalt concrete. The replacement rate was 0%, 15%, 30%, 45%, and 60% by weight of total mineral filler. Scanning electron microscopy (SEM) was employed to assess the surface morphology of Sawdust (SD), SDA, and LD. In addition, a series of tests, including Marshall stability and flow, indirect tensile strength,moisture susceptibility, and repeated uniaxial loading tests, were conducted to examine the performance characteristics of asphalt mixtures of diffe

A fixed firefighting system is a key component of fire safeguarding and reducing fire danger. It is installed as a permanent component in a structure to protect the entire or a portion of the building and its contents. The study aims to review the previous studies that deal with the evaluation of fire safety measures and their use in resolving problems associated with fire threats in buildings. For this reason, a number of previous studies in this field were reviewed compared with the NFPA code. The findings revealed that regulatory developments over the last several decades had created an atmosphere conducive to innovation. This has resulted in a growth in the number of fixed firefighting system types now obtainable. Th

   The potential application of granules of brick waste (GBW) as a low-cost sorbent for removal of Ni⁺²ions from aqueous solutions has been studied. The properties of GBW were determined through several tests such as X-Ray diffraction (XRD), Energy dispersive X-ray (EDX), Scanning electron microscopy (SEM), and BET surface area. In batch tests, the influence of several operating parameters including contact time, initial concentration, agitation speed, and the dose of GBW was investigated. The best values of these parameters that provided maximum removal efficiency of nickel (39.4%) were 1.5 hr, 50 mg/L, 250 rpm, and 1.8 g/100mL, respectively. The adsorption data obtained by batch experiments subjected to the Three i

Water supply networks are marred by serious risks of imperceptible pipeline leakage, posing ‎sustainability ‎and ‎performance ‎threats.  This article highlights the use of vibratory signal features to get around the drawbacks of traditional methods in a highly detailed framework for leak detection based on CatBoost. demonstrated excellent diagnostic performance and carried out a thorough test performance evaluation on five leakage configurations  . The expected system achieved an accuracy of 98.1% (variance (well within x/3% of expected):, beating traditional competitors such as Random Forest (97.3%) and Support Vector Machine (93.8%). ‎For example, the area under the receiver-operating characteristic curve was 0.995, in

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The increasing discharge of dye-containing wastewater has become a serious environmental problem, requiring efficient and sustainable treatment technologies. In this study, poultry litter–derived biochar (BC) was prepared via pyrolysis at 550 °C and subsequently modified with chitosan to produce a composite adsorbent (BC/CS) for the removal of Congo Red (CR) from aqueous solutions. The materials were characterized using SEM, BET, XRD, FTIR, and XPS analyses. BET results showed that chitosan modification slightly decreased the specific surface area from 8.28 to 8.18 m²/g and pore volume from 0.035 to 0.030 cm³ /g, while introducing abundant amine and hydroxyl functional groups on the surface. Maximum adsorption occurred at pH 3, w

This research work aims to the determination of molybdenum (VI) ion via the formation of peroxy molybdenum compounds which has red-brown colour with absorbance wave length at 455nm for the system of ammonia solution-hydrogen peroxide-molybdenum (VI) using a completely newly developed microphotometer based on the ON-Line measurement. Variation of responses expressed in millivolt. A correlation coefficient of 0.9925 for the range of 2.5-150 ?g.ml-1 with percentage linearity of 98.50%. A detection limit of 0.25 ?g.ml-1 was obtained. All physical and chemical variable were optimized interferences of cation and anion were studied classical method of measurement were done and compared well with newly on-line measurements. Application for the use